Venous thrombosis in children and adolescents with Hodgkin lymphoma in Sweden.

INTRODUCTION: Pediatric patients with Hodgkin lymphoma (HL) have several risk factors for venous thromboembolism (VTE). Although these patients are occasionally treated with thromboprophylaxis, no guidelines are implemented in Sweden. Scarce data from adult patients indicate an increased risk of VTE, but pediatric data is largely missing. Given the favorable overall survival of HL, there should reasonably be more focus on preventing complications. MATERIALS AND METHODS: We conducted a retrospective cohort study, including all patients registered in the Childhood Cancer Registry under the age of 18years diagnosed with HL between January 2005 and December 2015 in Sweden. RESULTS: Data was retrieved from the medical records of all 163 patients (100%) at six Swedish pediatric cancer centers. The incidence of VTE was 7.7% (symptomatic VTE 3.9%). The median follow-up was 3.4years (range 0.3-10.5). Only five patients (3.1%) were treated with thromboprophylaxis. All VTE events occurred in the older age category (11-17years) and all but one (92.7%) had a mediastinal mass. While the VTE did not significantly affect the treatment of HL, it caused increased morbidity and 2/12 developed a post-thrombotic syndrome. No significant risk factors for VTE were identified. CONCLUSIONS: VTE is a relatively common complication of HL and its treatment, causing increased acute and long-term morbidity. However, due to limited number of events we could not demonstrate risk-factors for VTE that would identify patients who might benefit from thromboprophylaxis.

Are children from Crete abandoning a Mediterranean diet?

INTRODUCTION: Mediterranean countries such as Greece have experienced rapid social change in the last decade. These community changes affect nutritional habits and there is a tendency for the traditional healthy Mediterranean diet to be abandoned. METHODS: The parents of children from one rural Greek village on Crete (Neapolis), and one rural village in Sweden (Kisa) were invited to their primary health care centers for an interview and to fill in a validated nutrition questionnaire, KidMed. RESULTS: There were no differences (p = 0.48) in total KidMed score between the Cretan and Swedish children, adjusted for gender and age. However, there were some significant differences in scores on certain KidMed questions. Parents of the Cretan children reported significantly higher daily use of olive oil at home and more regular nut consumption, but also more commercially baked goods or pastries for breakfast. The parents of Swedish children reported significantly higher use of cereals, grains or bread for breakfast. The mean BMIs were similar for the Cretan (Neapolis mean 16.8, 95% CI 13.5-23.0) and for the Swedish children (Kisa mean 17.4, 95% CI 13.7-25.5) CONCLUSION: The results suggest the possibility of changing nutritional habits, measurable among young children in rural areas. The study raises the question of whether Cretan children may have abandoned some aspects of the traditional Mediterranean diet. It may also be that Swedish children have changed their diet in favor of a more Mediterranean food choice. The major limitation of the study is the small sample size, and further, larger studies are warranted.

Successful extracorporeal membrane oxygenation in four children with malignant disease and severe Pneumocystis carinii pneumonia.

BACKGROUND: Toxic deaths due to intensified treatment are of increasing concern in view of the generally improved prognosis for children with malignancies. Pneumocystis carinii pneumonia (PCP) during immunosuppressive treatment in children with malignancies is a severe complication with a poor prognosis if mechanical ventilation is required. Extracorporeal membrane oxygenation (ECMO) is a recognized technique that provides temporary respiratory support for patients with intractable respiratory failure. PROCEDURE: We here report successful ECMO support in four children with PCP, secondary to chemotherapy-induced immunosuppression, in whom the respiratory situation deteriorated despite pharmacological treatment and mechanical ventilation. The duration of ECMO was 7-43 days. RESULTS: Three children with acute lymphoblastic leukemia recovered from their PCP to continue chemotherapy and are now in complete continuous remission. Their pulmonary function normalized completely during 6-12 months after ECMO therapy. One child with a primitive neuroectodermal tumor survived the PCP and was off ECMO for 11 days before succumbing to unrelated complications. CONCLUSIONS: We conclude that ECMO offers an important means of respiratory support in children with severe PCP that can also be adopted during treatment for malignant disease.

Reverse transcriptase polymerase chain reaction on fine needle aspirates for rapid detection of translocations in synovial sarcoma.

OBJECTIVE: To evaluate the utilization of fine needle aspiration (FNA) biopsy to obtain material for reverse-transcriptase polymerase chain reaction (RT-PCR) in the detection of the t(X;18)(p11.2;q11.2) translocation in synovial sarcomas. STUDY DESIGN: We applied RT-PCR to detection of synovial sarcoma fusion gene transcripts on fine needle aspirates. Five clinical samples were first analyzed: one was a tumor previously diagnosed as malignant hemangiopericytoma, one was a poorly defined tumor, and three were suspected synovial sarcomas. FNA material was transferred directly to the RT-PCR reaction tube without RNA extraction. RESULTS: The t(X;18) translocation could be detected on the limited amount of material that FNA provides. In each of the cases studied the representivity of the tumor samples was confirmed microscopically. CONCLUSION: Our protocol permits analysis directly on representative samples without extraction of RNA. The results imply that RT-PCR offers reliable detection of sarcoma fusion gene transcripts on fine needle aspirates. The procedure, apart from being applicable to outpatients, is rapid and sensitive.

Incidence of microalbuminuria in children with pyelonephritic scarring.

There is experimental evidence that loss of renal parenchyma results in hyperfiltration in the remnant glomeruli followed by development of glomerulosclerosis. Microalbuminuria, i.e., a urinary albumin excretion rate of 20-200 micrograms/min, is considered to be an early predictor of diabetic glomerulosclerosis. Hypothetically, increased urinary albumin excretion in patients with pyelonephritic scarring may also indicate glomerulosclerosis, with risk for future deterioration of renal function. This study was performed to determine the incidence of increased albumin excretion in children with mild to moderate pyelonephritic scarring, and to relate the information to glomerular filtration rate (GFR; clearance of inulin) and effective renal plasma flow (clearance of para-aminohippuric acid), as well as to the degree of scarring. The functional investigations were performed under water diuresis. Fifty-seven children, aged 1.7-17.9 years, with pyelonephritic renal scarring were included in the study. Nine young healthy adults were used as controls. The GFR was significantly lower in the children with pyelonephritic scarring than in the controls (median 93 ml/min per 1.73 m2, range 48-133 vs. 111 ml/min per 1.73 m2, range 89-121, P < 0.05), and the urine albumin excretion was significantly higher (median 20 micrograms/min per 100 ml GFR, range 0.8-170 vs. 9.2 micrograms/min per 100 ml GFR, range 3.3-21, P < 0.05). An inverse correlation was found between urine albumin excretion and GFR. Increased urine albumin excretion was found in 70% of the children with a GFR below 90 ml/min per 1.73 m2 compared with 41% of the children with a GFR above this level. Increased urine albumin excretion (> 20 micrograms/min per 100 ml GFR) was found in 51% of the children with pyelonephritic scarring, while only 14% had increased age-adjusted serum creatinine concentrations. The high incidence of microalbuminuria in children with pyelonephritic scarring indicates long-term follow-up until the ultimate outcome has been better defined.

Dermatofibrosarcoma protuberans of the vulva. A case report.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that is uncommon anywhere on the body but is especially uncommon on the vulva. This cancer rarely metastasizes but has a high incidence of local recurrence because of its tendency to grow in fingerlike projections away from the primary tumor. CASE: A 36-year-old, black woman was the 10th and youngest patient with DFSP of the vulva to be reported. Treatment of the 5-cm, mobile, well-circumscribed lesion consisted of local excision. Several adjacent microscopic lesions showing DFSP were found in the pathologic specimen, however, so an even wider excision was performed. Twenty-seven months following removal, there was no evidence of recurrence. CONCLUSION: Survival rates for DFSP of the skin range from 91% to 100% in reported series, but local recurrence rates of 20-49% have also been noted. The reported deaths from the disease have resulted from extensive local spread due to inadequate excision and, only rarely, metastases. Because DFSP tends to spread to microscopic projections away from the visible lesions, very wide local excision is required for tumor control.

Carcinoma of the endometrium.

Carcinoma of the endometrium is the most common gynecologic malignancy. The majority of women present with stage I disease, and the most common presenting symptom is postmenopausal bleeding. Early detection of endometrial cancer is important, because up to 90 percent of patients with stage I disease can be successfully treated. Certain risk factors, such as obesity, hypertension and diabetes mellitus, are associated with the development of this malignancy. Office endometrial sampling has a sensitivity of up to 97 percent for diagnosing carcinoma of the endometrium and can often eliminate the need for dilatation and curettage. Endometrial cancer is treated by total abdominal hysterectomy, bilateral salpingo-oophorectomy and, in many cases, postoperative radiation therapy.

Tubular response to hormones is blunted in weanling rats.

The renal responses to atrial natriuretic peptide (ANP), parathyroid hormone (PTH) and arginine vasopressin (AVP) were studied in anaesthetized weanling and adult rats with clearance methods. In rats receiving PTH, thyroparathyroidectomy (TPTX) was performed prior to the study. The results showed that GRF remained unchanged in both age groups during infusion with ANP, AVP and PTH after TPTX as well as with TPTX alone. During ANP infusion, the urine flow rate increased from 0.01 +/- 0.002 to 0.029 +/- 0.004 ml (100 g body wt)-1 min-1 (P less than 0.001) and the fractional sodium excretion increased from 0.48 +/- 0.1 to 3.0 +/- 0.4% (P less than 0.001) in the adult rats; no significant changes were observed in the weanling rats. Phosphate excretion was not influenced by ANP in either age group. After TPTX, both net and fractional phosphate excretion decreased in both age groups. During PTH infusion in TPTX rats, both net and fractional phosphate excretions increased significantly in the adult rats but were unchanged in the weanling rats. In rats of the same age, the renal concentrating capacity and urinary excretion of prostaglandin E2 (PGE2) were determined after dehydration. The renal concentrating capacity was lower, and the renal excretion of PGE2 was higher, in the weanling than in the adult rats. Furthermore, the concentrating mechanism seems not to be influenced by indomethacin treatment in either age group. It is concluded that the tubular responses to ANP and PTH are blunted in the immature kidney and that the renal excretion of PGE2 is not an important factor in the regulation of the concentrating capacity in the weanling rat.

Renal response to low and high phosphate intake in weanling, adolescent and adult rats.

The renal response to low and high phosphate intake was studied in weanling, young and adult rats. Weanling rats were started on experimental diets containing 0.37%, 0.7%, or 1.7% phosphate at 24 days and adult rats at 60 days of age. After 21 days, clearance studies were done in anaesthetized animals. Urine was collected during basal conditions and following a phosphate infusion. Urinary excretion of calcium, phosphate and creatinine, and plasma levels of phosphate and creatinine were determined. Plasma phosphate was slightly higher in the younger rats in all dietary groups but was not influenced by phosphate intake in either age group. Urinary phosphate excretion and fractional phosphate excretion increased significantly in both age groups with increasing phosphate intake. After high phosphate intake, both net and fractional phosphate excretions were significantly higher in younger rats (0.97 +/- 0.08 and 0.24 +/- 0.06 mumol min-1 100 g-1, P less than 0.01, and 47.5 +/- 3.84 and 18.15 +/- 5.59%, P less than 0.01, respectively). The urinary excretion of calcium related to creatinine was higher in younger rats in all dietary groups with the highest value found after low phosphate intake. During an acute phosphate infusion, fractional phosphate excretion increased significantly in both age groups after normal phosphate intake but remained unchanged after low or high phosphate intake. Plasma phosphate increased significantly only in younger rats with high phosphate intake (2.9 +/- 0.18, 3.88 +/- 0.43, P less than 0.05). It is suggested that hypercalciuria reflects early stages of phosphate depletion and that in young rats stabilized on a high phosphate intake, phosphate retention may occur during an acute phosphate load.

Renal response to parathyroid hormone in the weanling rat.

The renal response to parathyroid hormone and the activity of alkaline phosphatase in kidney cortex were studied in weanling and adult rats. Clearance studies for the determination of glomerular filtration rate and phosphate excretion were started 2 h after sham operation or thyroparathyroidectomy in 24- or 40-day-old rats. Urine was collected under basal conditions and following infusions with 0.5 and 1.0 mumol phosphate per 100 g body wt min-1. For enzyme studies, rats aged 14-40 days were used, and the activity of alkaline phosphatase in renal cortical slices was determined. Glomerular filtration rate was not influenced by thyroparathyroidectomy or phosphate infusions. Plasma phosphate increased during phosphate infusions and was significantly higher in thyroparathyroidectomized rats in all clearance periods. Both the net and the fractional excretions of phosphate were higher in intact than in thyroparathyroidectomized rats and the phosphate-retaining effect of thyroparathyroidectomy was similar in weanling and adult rats. Alkaline phosphatase activity in renal cortical slices increased between days 14 and 24 (1400-3190 IU mg-1 protein, P less than 0.01). During later development, no significant change was seen. It is concluded that the immature kidney of the weanling rats shows a parathyroid hormone-dependent adaptation to acute phosphate loading, and that changes in alkaline phosphatase activity are consistent with a maturation of this system only during early development.

[Decompressive craniotomy in severe craniocerebral trauma following unsuccessful treatment with barbiturates]. / Dekompressive Kraniotomie bei schwerem Schädelhirntrauma nach erfolgloser Behandlung mit Barbituraten.

During the period from September 1983 to October 1984 the intracranial pressure of 51 patients who had suffered cranio-cerebral trauma was continuously recorded. In 21 patients (41%) the intracranial pressure remained within normal limits (10-25 mm Hg) and apart from hyperventilation and corticosteroids no other treatment was required. All these patients recovered satisfactorily. In 28 patients the raised intracranial pressure could not be reduced by means of hyperventilation, corticosteroids and osmotherapy so that barbiturates had to be administered. Seven patients did not respond to this additional treatment and a decompression craniotomy was necessary. The progress, treatment, complications and prognosis of such patients are discussed.

Renal excretion of calcium and phosphate in preterm and term infants.

We studied urinary phosphate and calcium excretion in preterm and term infants during the first 3 months of life. The infants were mainly breast-fed, and the average phosphate intake ranged between 0.5 and 1 mmol/kg/day. During the first week of life urinary phosphate excretion was significantly higher in preterm than in term infants, whereas parathyroid hormone values were the same. After the first week of life urinary phosphate and calcium excretion were the same in preterm and term infants. Fractional excretion of phosphate was low (range 1% to 6%). In both groups calcium excretion was low during the first weeks of life, and increased thereafter to 5 and 3 mmol/1.73 m2/day, respectively. The urinary calcium/creatine ratio generally exceeded 2.0 (mmol/mmol) in preterm infants after the second week of life. These results are compatible with a state of relative phosphate deficiency, resulting in an adaptively low urinary phosphate excretion and an inability to form bone minerals, and therefore relatively high urinary calcium excretion.

The multidisciplinary team approach to exenteration of the pelvis.

The experience of a newly formed, multidisciplinary, pelvic exenteration team is presented which consists of a gynecologic oncologist, a general surgeon and a urologist. The first 20 exenterations performed by this team are reviewed. Nine patients had no postoperative problems, and of the 11 patients who did have complications, all but two, which resulted in death, were corrected without resultant debilitation. Fourteen of the 18 patients who survived the operation are tumor-free for as long as three years postoperatively. Operative proficiency as well as operative mortality and morbidity have certainly improved quite significantly as the team has gained experience.

Dysgerminoma associated with pregnancy.

From a review of 26 reported case (our case being the 27th) of dysgerminoma associated with pregnancy, several salient facts are evident. Torsion and incarceration are common among these rapidly enlarging tumors. Obstetrical complications occurred in nearly half and fetal demise in one quarter of the reviewed cases. Our case was typical of patients presenting with this vexed problems: The patient was under 25 (as were 70%); nulliparous (as were 67%); and the tumor appeared confined to one ovary (as in 89%). Authorities are in dispute as to the treatment of stage IA dysgerminomas, and the association of pregnancy complicates this debate even further. The results of conservative treatment in this series were jarring: There were recurrences in 30% of the 23 stage IA tumors, and the recurrences were all following unilateral oophorectomy. In our case, the grossly normal contralateral ovary was infiltrated with dysgerminoma cells. While treatment of a young woman with a dysgerminoma of 1 ovary is a matter of perplexity, we believe that a unilateral operation should be limited to those women who desire above all earthly things to retain their childbearing capacity.